Subcutaneous panniculitis-like T-cell lymphoma presented with full facial edema, hemophagocytic syndrome and Epstein-Barr Virus infection:A case report

Abstract

BackgroundSubcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare and distinct type of T-cell lymphoma. It is frequently associated with hemophagocytic syndrome (HPS), whose etiologic factors always involve Epstein-Barr Virus(EBV) infection. Although EBV is absent in many cases of sporadic SPTCL, we found a case of SPTCL with full facial edema, HPS and EBV infection as the first symptom. Patient and treatmentA 36-year-old female had presented with fever, full facial edema for 20 days before hospitalization. Remarkable abnormalities were found as hypersplenotrophy, pancytopenia, hypofibrinogenemia, high ferritin and soluble interleukin-2-receptor level, hemophagocytosis in bone marrow smear, which indicated HPS. Detection by PCR of EBV-DNA in peripheral blood was 9.73×104 copies. On the next day of her hospitalization, HLH-2004 protocol (VP-16+dexamethasone+cyclosporine A) was performed. Her temperature fell to normal on the 4th day. Full facial edema vanished gradually in the following days. After 8 weeks' treatment, all the abnormalities in the laboratory examination returned to normal. Despite of cyclosporine A and prednisone were used as continual treatment, facial edema and fever relapsed accompanied by multiple skin lesions on her back, upper and lower limbs 4 months later. Reexamination of the laboratory index related above were abnormal once again. Copy number of EBV-DNA was 5.52×105. Histologic and immunohistochemical features of tissue biopsy from the skin lesion on her right arm revealed the evidence of SPTCL. Then, she was treated by asparaginase plus DICE regimen. The facial edema and skin lesion vanished gradually during the course of treatment. Now (one month after the first course of chemotherapy), the patient is in her second course of asparaginase plus DICE regimen and SPTCL remains remission. ConclusionThis was not a typical case of SPTCL. The patient presented with overt skin lesion 4 months after the first symptom of full facial edema, which suggested SPTCL is a heterogenous disease with variable presentations. Awareness of this rare entity is critical to both ensure timely diagnosis and treatment. In addition, the pathogenesis of SPTCL remains unclear. Some malignant lymphomas are induced by chronic inflammation due to viral or bacterial infection. It is well known that EBV infection often causes malignant lymphomas of B-cell type, particularly in elderly peoples and immunocompromised individuals. Although EBV is absent in many cases of sporadic SPTCL, the virus appears to be implicated in the etiology of variant SPTCL occurring in the Asian population. Disclosures:No relevant conflicts of interest to declare.